{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T08:20:31Z","timestamp":1790670031106,"version":"4.1.0"},"reference-count":20,"publisher":"European Respiratory Society (ERS)","issue":"4","license":[{"start":{"date-parts":[[2019,2,6]],"date-time":"2019-02-06T00:00:00Z","timestamp":1549411200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.ersjournals.com\/user-licence"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["F32 HL-144145"],"award-info":[{"award-number":["F32 HL-144145"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["K24 HL-103844"],"award-info":[{"award-number":["K24 HL-103844"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["P01 AG032953-A1"],"award-info":[{"award-number":["P01 AG032953-A1"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["T32 HL-007891"],"award-info":[{"award-number":["T32 HL-007891"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2019,1,17]]},"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:p>A clinically useful model to prognose onset of respiratory insufficiency in amyotrophic lateral sclerosis (ALS) would inform disease interventions, communication and clinical trial design. We aimed to derive and validate a clinical prognostic model for respiratory insufficiency within 6\u2005months of presentation to an outpatient ALS clinic.<\/jats:p><jats:p>We used multivariable logistic regression and internal cross-validation to derive a clinical prognostic model using a single-centre cohort of 765 ALS patients who presented between 2006 and 2015. External validation was performed using the multicentre Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database with 7083 ALS patients. Predictors included baseline characteristics at first outpatient visit. The primary outcome was respiratory insufficiency within 6\u2005months, defined by initiation of noninvasive ventilation, forced vital capacity (FVC) &lt;50% predicted, tracheostomy, or death.<\/jats:p><jats:p>Of 765 patients in our centre, 300 (39%) had respiratory insufficiency or death within 6\u2005months. Six baseline characteristics (diagnosis age, delay between symptom onset and diagnosis, FVC, symptom onset site, amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) total score and ALSFRS-R dyspnoea score) were used to prognose the risk of the primary outcome. The derivation cohort c-statistic was 0.86 (95% CI 0.84\u20130.89) and internal cross-validation produced a c-statistic of 0.86 (95% CI 0.85\u20130.87). External validation of the model using the PRO-ACT cohort produced a c-statistic of 0.74 (95% CI 0.72\u20130.75).<\/jats:p><jats:p>We derived and externally validated a clinical prognostic rule for respiratory insufficiency in ALS. Future studies should investigate interventions on equivalent high-risk patients.<\/jats:p>","DOI":"10.1183\/13993003.02237-2018","type":"journal-article","created":{"date-parts":[[2019,2,7]],"date-time":"2019-02-07T05:25:15Z","timestamp":1549517115000},"page":"1802237","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":40,"title":["Development of a prognostic model of respiratory insufficiency or death in amyotrophic lateral sclerosis"],"prefix":"10.1183","volume":"53","author":[{"given":"Jason","family":"Ackrivo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1989-7244","authenticated-orcid":false,"given":"John","family":"Hansen-Flaschen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. Paul","family":"Wileyto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard\u00a0J.","family":"Schwab","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Elman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven M.","family":"Kawut","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2019,2,6]]},"reference":[{"key":"2024102100481006000_53.4.1802237.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1603471"},{"key":"2024102100481006000_53.4.1802237.2","doi-asserted-by":"publisher","DOI":"10.2188\/jea.JE20130045"},{"key":"2024102100481006000_53.4.1802237.3","doi-asserted-by":"crossref","first-page":"e0131965","DOI":"10.1371\/journal.pone.0131965","article-title":"Survival and cause of death among a cohort of confirmed amyotrophic lateral sclerosis cases","volume":"10","author":"Paulukonis","year":"2015","journal-title":"PLoS One"},{"key":"2024102100481006000_53.4.1802237.4","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1007\/s00415-010-5494-7","article-title":"Validating population-based registers for ALS: how accurate is death certification?","volume":"257","author":"Yeo","year":"2010","journal-title":"J Neurol"},{"key":"2024102100481006000_53.4.1802237.5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2009.01290.x"},{"key":"2024102100481006000_53.4.1802237.6","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(05)70326-4"},{"key":"2024102100481006000_53.4.1802237.7","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181bc0141"},{"key":"2024102100481006000_53.4.1802237.8","doi-asserted-by":"publisher","DOI":"10.7326\/M14-0697"},{"key":"2024102100481006000_53.4.1802237.9","doi-asserted-by":"publisher","DOI":"10.1080\/146608200300079536"},{"key":"2024102100481006000_53.4.1802237.10","first-page":"1586","article-title":"A longitudinal support vector regression for prediction of ALS score","volume":"2015","author":"Du","year":"2015","journal-title":"IEEE Int Conf Bioinform Biomed Workshops IEEE"},{"key":"2024102100481006000_53.4.1802237.11","doi-asserted-by":"crossref","first-page":"444","DOI":"10.3109\/21678421.2014.893361","article-title":"RandomForest4Life: a random forest for predicting ALS disease progression","volume":"15","author":"Hothorn","year":"2014","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2024102100481006000_53.4.1802237.12","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000194316.91908.8a"},{"key":"2024102100481006000_53.4.1802237.13","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.3051"},{"key":"2024102100481006000_53.4.1802237.14","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-015-7731-6"},{"key":"2024102100481006000_53.4.1802237.15","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/S1474-4422(18)30089-9","article-title":"Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model","volume":"17","author":"Westeneng","year":"2018","journal-title":"Lancet Neurol"},{"key":"2024102100481006000_53.4.1802237.16","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-006-0226-8"},{"key":"2024102100481006000_53.4.1802237.17","doi-asserted-by":"crossref","first-page":"310","DOI":"10.3109\/17482960802566824","article-title":"Prognostic factors in ALS: a critical review","volume":"10","author":"Chi\u00f2","year":"2009","journal-title":"Amyotrophic Lateral Sclerosis"},{"key":"2024102100481006000_53.4.1802237.18","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000049472.47709.3B"},{"key":"2024102100481006000_53.4.1802237.19","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.59.1.99"},{"key":"2024102100481006000_53.4.1802237.20","unstructured":"Allison PD . Measures of fit for logistic regression (paper 1485-2014). SAS Global Forum, Washington, DC, March 23-26, 2014. https:\/\/statisticalhorizons.com\/wp-content\/uploads\/GOFForLogisticRegression-Paper.pdf Date last accessed: March 15, 2018."}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/13993003.02237-2018","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T07:35:02Z","timestamp":1740382502000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/13993003.02237-2018"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2,6]]},"references-count":20,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2019,4,18]]},"published-print":{"date-parts":[[2019,4]]}},"alternative-id":["10.1183\/13993003.02237-2018"],"URL":"https:\/\/doi.org\/10.1183\/13993003.02237-2018","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2,6]]}}}